Ebstein's Anomaly

By: Mireya Hernandez and Rachel Bae

What is it?

Rare heart defect that is congenital. There is an abnormality in the tricuspid valve and blood leaks back into the right atrium when the right ventricle contracts. Two leaflets are the tricuspid valve are displaced downward into the pumping chamber. The third leaflet is elongated and may be tethered to the wall of the chamber.
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Who is Most Likely to get it?

It is congenital and the factors that cause it are still unknown. Severe cases can be most noticeable in babies, and mild cases may not cause symptoms until adulthood.


There around 100 babies in America who are born with this condition.

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SYMPTOMS

  • Shortness of breath, especially with exertion
  • Fatigue
  • Heart palpitations or abnormal heart rhythms (Arrhythmia)
  • A bluish discoloration of the lips and skin caused by low oxygen (Cyanosis)
  • Cough
  • Failure to grow
  • Rapid Breathing

HOW IS IT DIAGNOSED

Tests that can help diagnose Ebstein's Anomaly:
  • Chest x-ray
  • Magnetic resonance imaging (MRI) of the heart
  • <--- Ultrasound of the heart (echocardiogram)
  • Electrocardiogram (ECG or EKG) - measures the electrical activity of the heart and shows the heart's electrical activity as line tracings on paper
  • Holter monitor testing. - Longer ECG (could last days to weeks)
  • Exercise stress test - test used to provide information about how the heart responds to exertion
  • Cardiac catheterization. - A long, thin, flexible tube called a catheter is put into a blood vessel in your arm, groin (upper thigh), or neck and threaded to your heart. Contrast dye is put into the catheter and observed through an x-ray
  • Cardiovascular Autonomic Reflex Test - Your body’s response to different stimuli, shown by changes in blood pressure and heart rate, will help your doctor understand how your autonomic nervous system is working.
  • Laboratory Test
  • Nuclear Imaging- radioactive tracer materials are administered and the radiation is detected, then formed into an image.
  • Radiographic Test - short wavelength electromagnetic radiation used to create pictures of the internal structures of the chest.

Treatment Methods

Treatment is not always needed. It depends on how much oxygen is flowing through the body.


Heart rhythm disturbances --> medications may help control heart rate and maintain normal heart rhythm.


BUT at some point in time, surgery is often required because of heart complications such as heart failure.


Most common Surgery methods:

- Tricuspid Valve Repair

- Tricuspid Valve Replacement

- Closure of the Atrial Septal Defect (ASD): can close if there is a hole between atriums during surgery

- Maze Procedure: creates scar tissue to redirect electrical signal to a proper flow


Radiofrequency Ablation (RFA) can treat arrhythmia

- Removes tissue using the heat generated from frequency currents to direct electrical signals


More Severe cases may require a Heart Transplant




Below: Kieron Balfour, of Blantyre, taken following his heart surgery.

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Prognosis

Earlier symptoms develop --> more severe the disease.

Children diagnosed after one year tend to have a very good prognosis and can usually live a normal life.


Some may have either no symptoms or very mild symptoms.


Others may worsen over time --> developing blue coloring (cyanosis), heart failure, heart block, or dangerous heart rhythms.


POSSIBLE COMPLICATIONS


A severe leakage can lead to swelling of the heart and liver, and congestive heart failure.

  • Arrhythmia
  • Blood clots from the heart to other parts of the body
  • Brain abscess
Ebstein Anomaly